Glomerulonephritis - Goodpasture Syndrome

Question 1

Pathophysiology of Goodpasture Syndrome.

Answer 1

Anti-GBM antibodies (against Type IV Collagen) attack the glomerulus and pulmonary basement membranes.

Question 2

Clinical Features of Goodpasture Syndrome.

Answer 2

1. Glomerulonephritis : AKI.

2. Pulmonary Haemorrhage : Haemoptysis.

Question 3

Differential of Goodpasture Syndrome.

Answer 3

Granulomatosis with Polyangiitis - ANCA antibodies, Wheeze, Sinusitis, Saddle-Shaped Nose (haemoptysis + AKI).

Question 4

Investigations of Goodpasture Syndrome.

Answer 4

1. Renal Biopsy : Linear IgG Deposits along Basement Membrane.
2. Raised TF following Pulmonary Haemorrhage.

Question 5

Management of Goodpasture Syndrome (3).

Answer 5

1. Plasmapheresis (Plasma Exchange) : remove circulating antibodies.
2. Steroids and Cyclophosphamide to stop further production of antibodies.
3. Dialysis if Severe AKI.

Question 6

When may Renal Transplantation be indicated in ESRF?

Answer 6

Anti-GBM antibodies are undetectable for 12 months and disease is in remission for at least 6 months without cytotoxic agents.

Question 7

What factors can increase the likelihood of pulmonary haemorrhage? (5)

Answer 7

1. Smoking.
2. LRTI.
3. Pulmonary Oedema.
4. Inhalation of Hydrocarbons.
5. Young Males.